Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations

Abstract

Non-cirrhotic portal hypertension (NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias (hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure, especially in extra-hepatic portal vein obstruction (EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life (QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis (NCPF) is a less common cause of portal hypertension in children in developing nations. Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis (CHF) presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli’s disease, renal cysts, and syndromes associated with neurological defects. Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplantation is required when there is decompensation or recurrent cholangitis, especially in Caroli’s syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.

Keywords: Extrahepatic portal vein obstruction; Non-cirrhotic portal fibrosis; Portosystemic shunt surgery; Congenital hepatic fibrosis

Core Tip: The review discusses the natural history, endoscopic outcome, and management of non-cirrhotic causes of portal hypertension in children, especially in resource constraint developing nations. Extrahepatic portal vein obstruction is the most common cause of portal hypertension in developing countries. Endoscopic variceal ligation and sclerotherapy effectively eradicate the esophageal varices. Other complications require shunt surgery that ultimately reverses portal hypertension. Non-cirrhotic portal fibrosis has favourable outcomes in terms of variceal bleeding and mortality. Isolated congenital hepatic fibrosis (CHF) has a relatively good outcome. Liver transplantation is required when CHF is associated with Caroli’s disease, recurrent cholangitis, and decompensation. The presence of significant renal disease requires combined liver and kidney transplantation.